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Rapidly Progressive Dementias

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Imaging Dementia

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Abstract

Main imaging findings:

  • CT and MRI serial scans may show rapid atrophy progression, with ventricular enlargement and progressive cortical thinning.

  • MRI:

    • DWI represents the most accurate MRI sequence to reveal brain abnormalities in prion diseases.

    • Typical pattern: bilateral hyperintensities on T2-weighted, FLAIR, and DWI scans with cortical and basal ganglia involvement.

    • During disease progression, these intensity abnormalities may decrease or disappear on DWI studies.

    • Pulvinar Sign: confluent hyperintensity in the pulvinar and in the dorsomedial thalami on T2-weighted, FLAIR, and DWI scans; sensitive marker of variant CJD.

    • Double hockey-stick sign: confluent hyperintensity in the dorsomedial thalami on T2-weighted, FLAIR, and DWI scans; sensitive marker of variant CJD.

    • Absence of contrast enhancement.

  • FDG-PET can be used to show hypometabolism of the frontal cortex, thalamus, and caudate nucleus when other biomarkers (i.e., MRI, CSF, EEG) are not available.

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Authors and Affiliations

  1. Neuroimaging Research Unit, Division of Neuroscience, Neurology Unit, Neurorehabilitation Unit, Neurophysiology Service, IRCCS San Raffaele Scientific Institute, Milan, Italy

    Massimo Filippi

  2. Vita-Salute San Raffaele University, Milan, Italy

    Massimo Filippi & Federica Agosta

  3. Neuroimaging Research Unit, Neurology Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy

    Federica Agosta

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  1. Massimo Filippi
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Filippi, M., Agosta, F. (2021). Rapidly Progressive Dementias. In: Imaging Dementia. Springer, Cham. https://doi.org/10.1007/978-3-030-66773-3_5

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